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但室间隔缺损合并三尖瓣反流、左心室右心房分流、部分性或完全性房室间隔缺损、肺静脉异位引流至右心房或腔静脉或体循环动静脉瘘均可导致有心房血氧饱和度升高。 | [
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在大型房间隔缺损，左右心房的收缩压或平均压相等。 | [
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右心室压轻度上升，多在25～35mmHg之间，在少数患儿可有右心室压中度上升。 | [
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有时在右心室与肺动脉间可测到15～30mmHg的压力阶差。 | [
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肺动脉压力多正常或轻度增高。 | [
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通常情况下，肺动脉阻力在40Um<sub>2</sup>以下。 | [
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【治疗】（一）外科治疗对于绝大多数房间隔缺损患儿，即是症状很轻甚至无症状，仍然需要选择性外科治疗。 | [
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通常婴儿对房间隔缺损已有较好的耐受，故选择性手术时间多在2～4岁。 | [
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延迟手术并无任何裨益，如青春期后手术，长期的容量负荷过重可造成右心房、右心室某些不可逆的变化而导致房性心律失常甚至死亡。 | [
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如有合并心功能衰竭或肺动脉高压时应尽早手术。 | [
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（二）经导管封堵治疗自1976年King和Mills首先用双伞形补片装置成功关闭继发性房间隔缺损以来，经导管介入性治疗房间隔缺损（ASD）得到迅速发展，封堵装置先后经历了Rashkind双面伞、Lock蚌壳、Sideris可调纽扣式补片等，1997年AmplatzK推出的Amplatzer蘑菇状封堵器成为当前广泛使用的封堵装置。 | [
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而超声心动图在ASD经导管封堵治疗的术前筛查、术中监视及术后效果评价中起着重要作用。 | [
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封堵术的并发症有残余分流、装置结构折断、装置脱落栓塞等。 | [
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第十六篇神经肌肉疾病第一章儿童神经系统的解剖生理特点一、人类神经系统发育形成的主要过程人类神经系统结构和功能非常复杂。 | [
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据估计，人脑至少有1011</sup>个神经元。 | [
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神经元之间以错综复杂的联系形成神经网络，在突触处又有多种化学活性物质参与神经信息的传递。 | [
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神经系统调节人体所有的生理功能以及学习、记忆和思维等高级神经活动。 | [
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脑的正常发育对以后神经系统的结构和功能至关重要。 | [
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传统的观点认为神经元代表神经系统主要结构单位，而胶质细胞的作用被降低至被动地位。 | [
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近年大量研究证实，胶质细胞可对神经元发育与功能起到多方面的调控作用。 | [
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神经元与胶质细胞的动态相互作用已被认为是神经系统的基础功能单位。 | [
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在发育过程中遗传与环境因素共同作用而产生神经细胞存活与死亡的平衡，控制其过度增殖，从而保证各型神经细胞应有的数量。 | [
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在无脊椎动物，其中枢神经系统（CNS）相对简单，生命周期较短，因此其发育主要受遗传因素所指导。 | [
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脊椎动物CNS发育则反映了遗传/环境作用的连续性，环境因素对神经系统的发育产生了更多的影响。 | [
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人类神经系统发育的主要程序包括诱导及原始神经胚形成（发生高峰在妊娠3～4周）、前脑发育（发生高峰在2～3个月）、神经细胞增殖与分化（发生高峰在妊娠3～4个月），以及神经细胞移行与分化（发生高峰于妊娠3～5个月），神经组织过程包括突触连结及神经回路建立、树突发芽、膜兴奋性形成成（发生高峰于妊娠5个月～出生后数年）以及髓鞘化（发生高峰在出生～出生后数年）。 | [
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（一）诱导及神经胚形成人类胚胎发育的第3周，外胚层在脊索中胚层的诱导下分化为神经外胚层。 | [
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神经外胚层通过细胞增殖、增厚而形成神经板（neuralplate），进而向内凹陷形成神经沟，神经沟闭合形成神经管（neuraltube）沿神经板内陷的每侧边缘处出现神经嵴神经管在其内不断升高的液体压力作用下，前端膨胀形成三个囊逐渐形成前、中、后脑中枢神经系统和周围神经系统的一部分。 | [
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"entity": "前、中、后脑",
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神经嵴细胞在神经管背中线处起源，在神经管闭合时或稍后即开始移行，逐渐分化成脑神经节、脊神经节和自主神经系统。 | [
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（二）前脑发育（proencephalicdevelopment）前脑发育的高峰期为妊娠2～3个月，主要包括以下过程：①前脑形成：开始于神经管头端，自妊娠1个月末前神经孔闭合时开始。 | [
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②前脑分裂：高峰期为妊娠5～6周，主要包括水平方向形成一对视囊、嗅球及嗅径横贯分开端脑及间脑前脑中线发育：高峰期在妊娠2～3个月后，是形成胼胝体、透明隔、视交叉及下丘脑结构的重要基础。 | [
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"entity": "前脑",
"start_offset": 1,
"end_offset": 3,
"label": "bod"
},
{
"id": 1,
"entity": "视囊",
"start_offset": 29,
"end_offset": 31,
"label": "bod"
},
{
"id": 2,
"entity": "嗅球",
"start_offset": 32,
"end_offset": 34,
"label": "bod"
},
{
"id": 3,
"entity": "嗅径",
"start_offset": 35,
"end_offset": 37,
"label": "bod"
},
{
"id": 4,
"entity": "水平方向形成一对视囊、嗅球及嗅径",
"start_offset": 21,
"end_offset": 37,
"label": "sym"
},
{
"id": 5,
"entity": "端脑",
"start_offset": 41,
"end_offset": 43,
"label": "bod"
},
{
"id": 6,
"entity": "间脑",
"start_offset": 44,
"end_offset": 46,
"label": "bod"
},
{
"id": 7,
"entity": "横贯分开端脑及间脑",
"start_offset": 37,
"end_offset": 46,
"label": "sym"
},
{
"id": 8,
"entity": "前脑中线",
"start_offset": 46,
"end_offset": 50,
"label": "bod"
},
{
"id": 9,
"entity": "胼胝体",
"start_offset": 69,
"end_offset": 72,
"label": "bod"
},
{
"id": 10,
"entity": "透明隔",
"start_offset": 73,
"end_offset": 76,
"label": "bod"
},
{
"id": 11,
"entity": "视交叉",
"start_offset": 77,
"end_offset": 80,
"label": "bod"
},
{
"id": 12,
"entity": "下丘脑",
"start_offset": 81,
"end_offset": 84,
"label": "bod"
}
] |
（三）神经细胞增殖（proliferation）与分化（differentiation）人类胚胎发育的第3～4个月是神经元增殖的主要时期，此后至出生1年小脑外颗粒层仍在继续增殖，其他神经元出生后则已停止增殖。 | [
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"id": 0,
"entity": "神经细胞",
"start_offset": 3,
"end_offset": 7,
"label": "bod"
},
{
"id": 1,
"entity": "神经元",
"start_offset": 58,
"end_offset": 61,
"label": "bod"
},
{
"id": 2,
"entity": "小脑外颗粒层",
"start_offset": 76,
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"label": "bod"
},
{
"id": 3,
"entity": "神经元",
"start_offset": 91,
"end_offset": 94,
"label": "bod"
}
] |
（四）神经细胞的移行（migration）与分化在神经系统发育过程中，神经细胞往往从其“出生地”出发，经过长短不等的路程，迁移到预定的位置。 | [
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{
"id": 1,
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{
"id": 2,
"entity": "神经细胞",
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] |
这对于从一个薄壁的神经管演化成结构复杂的脑是十分必要的。 | [
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"entity": "神经管",
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{
"id": 1,
"entity": "脑",
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"end_offset": 21,
"label": "bod"
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] |
神经元移行发生高峰出现于妊娠3～5个月。 | [
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] |
这一时期的遗传和环境的任何异常因素均可导致神经元移行障碍，例如脑裂畸形（schizencephaly）、无脑回（lissencephaly）、多小脑回（polymicrogyria）以及灰质异位（heterotopia），临床常表现为智力和运动发育障碍及惊厥发作。 | [
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"entity": "神经元移行障碍",
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{
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{
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"entity": "多小脑回",
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"label": "dis"
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{
"id": 6,
"entity": "polymicrogyria",
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"label": "dis"
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{
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"entity": "灰质异位",
"start_offset": 93,
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"label": "dis"
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{
"id": 8,
"entity": "heterotopia",
"start_offset": 98,
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"label": "dis"
},
{
"id": 9,
"entity": "智力和运动发育障碍及惊厥发作",
"start_offset": 117,
"end_offset": 131,
"label": "dis"
}
] |
随着影像诊断学技术进步，尤其是高分辨磁共振技术发展，皮层发育不良已经成为儿童难治性癫痫的重要原因。 | [
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"entity": "影像诊断学",
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{
"id": 1,
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"entity": "皮层发育不良",
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},
{
"id": 3,
"entity": "儿童难治性癫痫",
"start_offset": 36,
"end_offset": 43,
"label": "dis"
}
] |
（五）脑发育的组织过程（organization）正常脑发育的组织过程十分复杂，包括板层结构形成、突触连结及神经回路建立、神经突起生长发芽、膜兴奋性形成成以及胶质细胞增殖分化等。 | [
{
"id": 0,
"entity": "脑",
"start_offset": 3,
"end_offset": 4,
"label": "bod"
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{
"id": 1,
"entity": "脑",
"start_offset": 27,
"end_offset": 28,
"label": "bod"
},
{
"id": 2,
"entity": "板层结构形成",
"start_offset": 42,
"end_offset": 48,
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},
{
"id": 3,
"entity": "膜兴奋性形成",
"start_offset": 70,
"end_offset": 76,
"label": "sym"
}
] |
脑发育的组织过程起始于妊娠5个月，直至出生后数年仍在继续。 | [
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] |
脑发育的组织过程可因多种因素引起异常，造成各类发育性脑病。 | [
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"entity": "脑",
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{
"id": 1,
"entity": "发育性脑病",
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"label": "dis"
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] |
例如智力低下及婴儿孤独症等。 | [
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"entity": "智力低下",
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{
"id": 1,
"entity": "婴儿孤独症",
"start_offset": 7,
"end_offset": 12,
"label": "dis"
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] |
（六）髓鞘化（myelination）人类神经系统的髓鞘化在出生后即开始，在出生后前8个月最快，可持续至生后数年。 | [
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{
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{
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"entity": "髓鞘化",
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] |
第七节囊性纤维化囊性纤维化（cysticfibrosis，CF）是一种常染色体隐性遗传性疾病。 | [
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"entity": "囊性纤维化",
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{
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{
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{
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"entity": "CF",
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{
"id": 4,
"entity": "常染色体",
"start_offset": 35,
"end_offset": 39,
"label": "bod"
}
] |
这一基因编码一种1480个氨基酸的蛋白质，称为CF跨膜调节蛋白（CFtransmembraneregulator，CFTR）。 | [
{
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"entity": "蛋白质",
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{
"id": 1,
"entity": "CF跨膜调节蛋白",
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{
"id": 2,
"entity": "CFtransmembraneregulator",
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"label": "bod"
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{
"id": 3,
"entity": "CFTR",
"start_offset": 57,
"end_offset": 61,
"label": "bod"
}
] |
后者表达于气道、胃肠道（包括胰、胆道系统）、汗腺、泌尿生殖道上皮，具有离子通道和调节功能。 | [
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"entity": "气道",
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{
"id": 1,
"entity": "胃肠道",
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{
"id": 2,
"entity": "胰、胆道系统",
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{
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"entity": "汗腺",
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{
"id": 4,
"entity": "泌尿生殖道上皮",
"start_offset": 25,
"end_offset": 32,
"label": "bod"
}
] |
CF基因突变可能影响上皮细胞Cl<sup>-</sup>分泌，而Na<sup>+</sup>吸收过量，导致气道表面水分不足，分泌物变得干燥黏稠、不易排出，因而易发生金黄色葡萄球菌和铜绿假单胞菌的呼吸道定植和继发感染。 | [
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"entity": "CF基因突变",
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{
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{
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{
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"entity": "分泌物",
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{
"id": 4,
"entity": "金黄色葡萄球菌",
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{
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"entity": "铜绿假单胞菌",
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"entity": "呼吸道",
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{
"id": 7,
"entity": "感染",
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"end_offset": 107,
"label": "dis"
}
] |
全身黏液分泌腺阻塞扩张，继而萎缩纤维变性。 | [
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"entity": "黏液分泌腺",
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{
"id": 1,
"entity": "萎缩纤维变性",
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] |
由于胰腺外分泌不足，导致脂肪吸收障碍和营养不良。 | [
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"entity": "胰腺",
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{
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"entity": "营养不良",
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] |
新生儿可由于胰液消化力弱，致胎粪呈硬性灰白色油状物，引起胎粪性肠梗阻。 | [
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{
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{
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"entity": "胎粪性肠梗阻",
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"label": "dis"
}
] |
由于汗腺导管的功能是吸收Cl<sup>-</sup>而不是分泌Cl<sup>-</sup>，因而CF患儿汗液中Cl<sup>-</sup>重吸收障碍，导致汗液中Cl<sup>-</sup>和Na<sup>+</sup>水平升高。 | [
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"entity": "汗腺导管",
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{
"id": 1,
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"start_offset": 48,
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{
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"entity": "汗液",
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{
"id": 3,
"entity": "汗液",
"start_offset": 77,
"end_offset": 79,
"label": "bod"
}
] |
本病是引起儿童慢性严重肺部疾病的主要原因，并与胰腺外分泌功能不全有关。 | [
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"entity": "慢性严重肺部疾病",
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{
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"entity": "胰腺外分泌功能不全",
"start_offset": 23,
"end_offset": 32,
"label": "dis"
}
] |
临床上以气道梗阻和感染及消化不良为特征。 | [
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{
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] |
根据美国20000例CF患者的调查，表现急性或持续呼吸道症状者占50.5%，生长发育障碍、营养不良者占42.9%，大便异常（如脂肪泻）占35.0%，胎粪性肠梗阻为18.8%，有家族史者占16.8%。 | [
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{
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"entity": "胎粪性肠梗阻",
"start_offset": 74,
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"label": "dis"
}
] |
有些患儿可长时间无症状，或仅有反复肺炎或急性呼吸道感染迁延不愈。 | [
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"entity": "肺炎",
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{
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"entity": "急性呼吸道感染",
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"label": "dis"
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] |
婴儿常闻哮鸣音。 | [
{
"id": 0,
"entity": "哮鸣音",
"start_offset": 4,
"end_offset": 7,
"label": "sym"
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] |
婴儿反复呼吸道感染若伴有消化不良，须考虑本病之可能。 | [
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"entity": "呼吸道感染",
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{
"id": 1,
"entity": "消化不良",
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] |
用毛果芸香碱电离子透入法收集并测定汗液中Cl<sup>-</sup>含量，如超过60mmol/L，结合下列一项或一项以上可诊断本病：典型慢性阻塞性肺疾病，胰腺外分泌功能不全，阳性家族史。 | [
{
"id": 0,
"entity": "毛果芸香碱电离子透入法",
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"end_offset": 12,
"label": "pro"
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{
"id": 1,
"entity": "汗液",
"start_offset": 17,
"end_offset": 19,
"label": "bod"
},
{
"id": 2,
"entity": "慢性阻塞性肺疾病",
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"end_offset": 76,
"label": "dis"
},
{
"id": 3,
"entity": "胰腺外分泌功能不全",
"start_offset": 77,
"end_offset": 86,
"label": "dis"
}
] |
近年来推荐新的诊断标准：典型临床特征（呼吸道症状、胃肠道、泌尿生殖系统症状），或同胞中有CF患儿，或新生儿筛查试验阳性，加下列CFTR功能紊乱的实验室证据之一即可诊断：两个不同日期采集的汗液Cl<sup>-</sup>含量升高，鉴定出两种CF突变，鼻上皮电位差测定异常。 | [
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{
"id": 1,
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{
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{
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"entity": "CF",
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{
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"entity": "新生儿筛查试验",
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{
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{
"id": 6,
"entity": "汗液",
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},
{
"id": 7,
"entity": "CF突变",
"start_offset": 119,
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"label": "dis"
},
{
"id": 8,
"entity": "鼻上皮电位差测定",
"start_offset": 124,
"end_offset": 132,
"label": "ite"
}
] |
设法去除呼吸道内黏稠分泌物，加强肺部物理治疗，尤其是胸部叩打和体位引流，根据病情每天1～4次；注意口服补液或静脉补液，尤其是炎热季节及伴发急性胃肠炎时，防止脱水和气道分泌物干燥黏稠；雾化吸入0.45～0.9%氯化钠可湿化气道、稀化痰液，亦可加用人重组DNA酶（2.5mg，每天1次）雾化吸入。 | [
{
"id": 0,
"entity": "呼吸道",
"start_offset": 4,
"end_offset": 7,
"label": "bod"
},
{
"id": 1,
"entity": "黏稠分泌物",
"start_offset": 8,
"end_offset": 13,
"label": "sym"
},
{
"id": 2,
"entity": "肺部物理治疗",
"start_offset": 16,
"end_offset": 22,
"label": "pro"
},
{
"id": 3,
"entity": "胸部叩打",
"start_offset": 26,
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"label": "pro"
},
{
"id": 4,
"entity": "体位引流",
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"label": "pro"
},
{
"id": 5,
"entity": "口服补液",
"start_offset": 49,
"end_offset": 53,
"label": "pro"
},
{
"id": 6,
"entity": "静脉补液",
"start_offset": 54,
"end_offset": 58,
"label": "pro"
},
{
"id": 7,
"entity": "急性胃肠炎",
"start_offset": 69,
"end_offset": 74,
"label": "dis"
},
{
"id": 8,
"entity": "脱水",
"start_offset": 78,
"end_offset": 80,
"label": "sym"
},
{
"id": 9,
"entity": "气道分泌物干燥黏稠",
"start_offset": 81,
"end_offset": 90,
"label": "sym"
},
{
"id": 10,
"entity": "雾化吸入",
"start_offset": 91,
"end_offset": 95,
"label": "pro"
},
{
"id": 11,
"entity": "氯化钠",
"start_offset": 104,
"end_offset": 107,
"label": "dru"
},
{
"id": 12,
"entity": "气道",
"start_offset": 110,
"end_offset": 112,
"label": "bod"
},
{
"id": 13,
"entity": "痰液",
"start_offset": 115,
"end_offset": 117,
"label": "bod"
},
{
"id": 14,
"entity": "人重组DNA酶",
"start_offset": 122,
"end_offset": 129,
"label": "dru"
},
{
"id": 15,
"entity": "雾化吸入",
"start_offset": 141,
"end_offset": 145,
"label": "pro"
}
] |
N-乙酰半胱氨酸可溶解黏液，但可损害纤毛上皮，应避免反复使用。 | [
{
"id": 0,
"entity": "N-乙酰半胱氨酸",
"start_offset": 0,
"end_offset": 8,
"label": "dru"
},
{
"id": 1,
"entity": "纤毛上皮",
"start_offset": 18,
"end_offset": 22,
"label": "bod"
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] |
伴可逆性气道梗阻者可应用β受体兴奋剂或色苷酸钠、盐酸异丙托品。 | [
{
"id": 0,
"entity": "可逆性气道梗阻",
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{
"id": 1,
"entity": "β受体兴奋剂",
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{
"id": 2,
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{
"id": 3,
"entity": "盐酸异丙托品",
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"end_offset": 30,
"label": "dru"
}
] |
适当放宽抗生素应用指征，剂量为一般轻症感染的2～3倍。 | [
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"entity": "抗生素",
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{
"id": 1,
"entity": "轻症感染",
"start_offset": 17,
"end_offset": 21,
"label": "dis"
}
] |
轻症感染可口服给药，主要针对常见病原如金黄色葡萄球菌、不定型流感嗜血杆菌、铜绿假单胞菌等，最好根据细菌培养和药物敏感试验结果选用。 | [
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"id": 0,
"entity": "轻症感染",
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{
"id": 1,
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{
"id": 2,
"entity": "金黄色葡萄球菌",
"start_offset": 19,
"end_offset": 26,
"label": "mic"
},
{
"id": 3,
"entity": "不定型流感嗜血杆菌",
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"label": "mic"
},
{
"id": 4,
"entity": "铜绿假单胞菌",
"start_offset": 37,
"end_offset": 43,
"label": "mic"
},
{
"id": 5,
"entity": "细菌培养",
"start_offset": 49,
"end_offset": 53,
"label": "ite"
},
{
"id": 6,
"entity": "药物敏感试验",
"start_offset": 54,
"end_offset": 60,
"label": "ite"
}
] |
严重感染者应静脉给药，疗程不少于2周。 | [
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"entity": "感染",
"start_offset": 2,
"end_offset": 4,
"label": "dis"
},
{
"id": 1,
"entity": "静脉给药",
"start_offset": 6,
"end_offset": 10,
"label": "pro"
}
] |
为控制炎症过程，延缓肺部病变的发展，有人提出应用皮质激素吸入治疗，但迄今尚缺乏肯定有效的证据，常规应用仅限于伴有严重反应性气道疾病者。 | [
{
"id": 0,
"entity": "炎症",
"start_offset": 3,
"end_offset": 5,
"label": "dis"
},
{
"id": 1,
"entity": "肺部病变",
"start_offset": 10,
"end_offset": 14,
"label": "sym"
},
{
"id": 2,
"entity": "皮质激素吸入治疗",
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"end_offset": 32,
"label": "pro"
},
{
"id": 3,
"entity": "严重反应性气道疾病",
"start_offset": 56,
"end_offset": 65,
"label": "dis"
}
] |
非甾体类抗炎药物如布洛芬可用于肺部病变较轻、一秒用力呼气量（FEV<sub>1</sub>）在预计值60%以上的5～12岁儿童。 | [
{
"id": 0,
"entity": "非甾体类抗炎药物",
"start_offset": 0,
"end_offset": 8,
"label": "dru"
},
{
"id": 1,
"entity": "布洛芬",
"start_offset": 9,
"end_offset": 12,
"label": "dru"
},
{
"id": 2,
"entity": "肺部病变",
"start_offset": 15,
"end_offset": 19,
"label": "sym"
},
{
"id": 3,
"entity": "一秒用力呼气量",
"start_offset": 22,
"end_offset": 29,
"label": "ite"
},
{
"id": 4,
"entity": "FEV<sub>1</sub>",
"start_offset": 30,
"end_offset": 45,
"label": "ite"
}
] |
此外，应给予高热量、高蛋白饮食，并注意补充多种维生素。 | [
{
"id": 0,
"entity": "高热量、高蛋白饮食",
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},
{
"id": 1,
"entity": "维生素",
"start_offset": 23,
"end_offset": 26,
"label": "dru"
}
] |
一些新的治疗手段目前正在积极研究中，如基因治疗、突变CFTR的药物上调、刺激其他Cl<sup>-</sup>转运机制等。 | [
{
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{
"id": 1,
"entity": "突变CFTR",
"start_offset": 24,
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] |
第五章小儿艾滋病小儿艾滋病即小儿时期的获得性免疫缺陷综合征（acquiredimmunodeficiencysyndrome，AIDS），由人类免疫缺陷病毒（humanimmunodeficiencyvirus，HIV）引起。 | [
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{
"id": 1,
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{
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"entity": "小儿时期的获得性免疫缺陷综合征",
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{
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"entity": "acquiredimmunodeficiencysyndrome",
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"label": "dis"
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{
"id": 4,
"entity": "AIDS",
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{
"id": 5,
"entity": "人类免疫缺陷病毒",
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"label": "dis"
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{
"id": 6,
"entity": "humanimmunodeficiencyvirus",
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"end_offset": 105,
"label": "mic"
},
{
"id": 7,
"entity": "HIV",
"start_offset": 106,
"end_offset": 109,
"label": "mic"
}
] |
AIDS为目前人类最为严重的传染病之一，小儿AIDS发生率的增长比成人快，每年约有40万儿童新感染HIV，截至2000年，全球约有800万儿童为HIV病毒携带者或AIDS患者。 | [
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"id": 0,
"entity": "AIDS",
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"end_offset": 4,
"label": "dis"
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{
"id": 1,
"entity": "小儿AIDS",
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"label": "dis"
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{
"id": 2,
"entity": "HIV",
"start_offset": 49,
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"label": "mic"
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{
"id": 3,
"entity": "HIV病毒",
"start_offset": 72,
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"label": "mic"
},
{
"id": 4,
"entity": "AIDS",
"start_offset": 81,
"end_offset": 85,
"label": "dis"
}
] |
这些受HIV感染的儿童大部分出生在发展中国家，而在北美及西欧，新生婴儿HIV病毒的感染率近年已明显下降。 | [
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"entity": "HIV感染",
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{
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"label": "mic"
},
{
"id": 2,
"entity": "病毒",
"start_offset": 38,
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"label": "mic"
}
] |
儿童HIV感染后的病理进程较成人更快，一些儿童可在2岁内因AIDS死亡，这是因为AIDS儿童的病毒负荷较成人大，被感染的CD4+</sup>T淋巴细胞（CD4+</sup>Tlymphocyte）在体内消耗的速度较成人更快的缘故。 | [
{
"id": 0,
"entity": "HIV感染",
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{
"id": 1,
"entity": "AIDS",
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"label": "dis"
},
{
"id": 2,
"entity": "AIDS",
"start_offset": 40,
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"label": "dis"
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{
"id": 3,
"entity": "病毒",
"start_offset": 47,
"end_offset": 49,
"label": "mic"
},
{
"id": 4,
"entity": "T淋巴细胞",
"start_offset": 70,
"end_offset": 75,
"label": "bod"
}
] |
我国目前已成为HIV感染的高发地区。 | [
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"entity": "HIV感染",
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"label": "dis"
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] |
1998年年初的统计表明，我国HIV感染者为9970人。 | [
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] |
至2002年年初，HIV感染者已急剧上升为80余万人，其中约40万人为AIDS现症病人。 | [
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"entity": "HIV感染",
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{
"id": 1,
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"end_offset": 39,
"label": "dis"
}
] |
因此小儿AIDS的防治，已成为儿科学界新的严峻挑战。 | [
{
"id": 0,
"entity": "AIDS",
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] |
【病原和传播途径】（一）病原HIV属于反转录病毒科，慢病毒属中的灵长类免疫缺陷病毒亚属。 | [
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"id": 0,
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{
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"end_offset": 24,
"label": "mic"
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{
"id": 2,
"entity": "慢病毒属",
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"entity": "灵长类免疫缺陷病毒亚属",
"start_offset": 32,
"end_offset": 43,
"label": "mic"
}
] |
目前已发现的HIV有两型，即HIV-1和HIV-2。 | [
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"label": "mic"
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{
"id": 1,
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"label": "mic"
},
{
"id": 2,
"entity": "HIV-2",
"start_offset": 20,
"end_offset": 25,
"label": "mic"
}
] |
HIV-1是引起全球性AIDS蔓延的主要病原体，而HIV-2的流行主要局限西非地区。 | [
{
"id": 0,
"entity": "HIV-1",
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{
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"label": "dis"
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{
"id": 2,
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] |
HIV病毒颗粒是一个直径大约为100nm的圆球体，核心有两个单股正链RNA和由病毒编码的p17、p24、p7及p6等核蛋白构成，病毒最外面的包膜则由病毒编码的糖蛋白gp120和gp41等以及类脂组成。 | [
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"entity": "HIV病毒颗粒",
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{
"id": 1,
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{
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{
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{
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{
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"label": "mic"
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{
"id": 6,
"entity": "糖蛋白gp120",
"start_offset": 79,
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"label": "bod"
},
{
"id": 7,
"entity": "gp41",
"start_offset": 88,
"end_offset": 92,
"label": "bod"
}
] |
（二）传播途径流行病学研究证实，HIV的传播主要通过三个途径：1.性传播即通过同性及异性间的性接触传播。 | [
{
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"end_offset": 19,
"label": "mic"
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] |
为HIV的主要传播途径，全球70%～80%的HIV感染是通过性传播的。 | [
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"id": 0,
"entity": "HIV",
"start_offset": 1,
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{
"id": 1,
"entity": "HIV感染",
"start_offset": 22,
"end_offset": 27,
"label": "dis"
}
] |
2.静脉药瘾者共用注射器传播输血和血液制品传播以及移植手术和针刺意外传播等。 | [
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"id": 0,
"entity": "注射器",
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"label": "equ"
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{
"id": 1,
"entity": "输血",
"start_offset": 14,
"end_offset": 16,
"label": "pro"
},
{
"id": 2,
"entity": "血液制品",
"start_offset": 17,
"end_offset": 21,
"label": "dru"
},
{
"id": 3,
"entity": "移植手术",
"start_offset": 25,
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"label": "pro"
},
{
"id": 4,
"entity": "针刺",
"start_offset": 30,
"end_offset": 32,
"label": "pro"
}
] |
3.母婴传播即垂直传播为小儿感染HIV的主要途径，其传播率为22%～65%。 | [
{
"id": 0,
"entity": "小儿感染HIV",
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"label": "dis"
}
] |
垂直传播途径：①胎儿期经胎盘传播；②分娩中经母亲的血液和分泌物传播；③分娩后经母乳传播。 | [
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"id": 0,
"entity": "胎盘",
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"label": "bod"
},
{
"id": 1,
"entity": "血液",
"start_offset": 25,
"end_offset": 27,
"label": "bod"
}
] |
其中以宫内感染为多见。 | [
{
"id": 0,
"entity": "宫内感染",
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"label": "dis"
}
] |
【发病机制】HIV致病的中心环节，是选择性地使CD4+</sup>T淋巴细胞大量消耗，导致免疫功能缺陷。 | [
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"id": 0,
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"start_offset": 6,
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"label": "mic"
},
{
"id": 1,
"entity": "T淋巴细胞",
"start_offset": 33,
"end_offset": 38,
"label": "bod"
}
] |
当HIV侵入人体后，首先识别CD4+</sup>T淋巴细胞和巨噬细胞，由病毒外膜的包膜蛋白gp120与细胞表面的CD4受体及辅助受体结合，使gp120构象改变，暴露出与细胞趋化因子受体结合的位点，并与之作用，从而介导HIV对CD4+</sup>细胞的吸附。 | [
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"id": 0,
"entity": "HIV",
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{
"id": 1,
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},
{
"id": 2,
"entity": "巨噬细胞",
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"label": "bod"
},
{
"id": 3,
"entity": "病毒",
"start_offset": 36,
"end_offset": 38,
"label": "mic"
},
{
"id": 4,
"entity": "包膜蛋白gp120",
"start_offset": 41,
"end_offset": 50,
"label": "bod"
},
{
"id": 5,
"entity": "CD4受体",
"start_offset": 56,
"end_offset": 61,
"label": "bod"
},
{
"id": 6,
"entity": "辅助受体",
"start_offset": 62,
"end_offset": 66,
"label": "bod"
},
{
"id": 7,
"entity": "gp120",
"start_offset": 70,
"end_offset": 75,
"label": "bod"
},
{
"id": 8,
"entity": "细胞趋化因子受体",
"start_offset": 84,
"end_offset": 92,
"label": "bod"
},
{
"id": 9,
"entity": "HIV",
"start_offset": 108,
"end_offset": 111,
"label": "mic"
}
] |
引起病毒脱衣壳，病毒核心部分进入细胞，在反转录酶的作用下，病毒的RNA转录为双链DNA，双链DNA在整合酶作用下与宿主细胞染色体DNA整合并形成前病毒DNA，随染色体复制而复制。 | [
{
"id": 0,
"entity": "病毒",
"start_offset": 2,
"end_offset": 4,
"label": "mic"
},
{
"id": 1,
"entity": "病毒",
"start_offset": 8,
"end_offset": 10,
"label": "mic"
},
{
"id": 2,
"entity": "细胞",
"start_offset": 16,
"end_offset": 18,
"label": "bod"
},
{
"id": 3,
"entity": "反转录酶",
"start_offset": 20,
"end_offset": 24,
"label": "bod"
},
{
"id": 4,
"entity": "病毒",
"start_offset": 29,
"end_offset": 31,
"label": "mic"
},
{
"id": 5,
"entity": "RNA",
"start_offset": 32,
"end_offset": 35,
"label": "bod"
},
{
"id": 6,
"entity": "双链DNA",
"start_offset": 38,
"end_offset": 43,
"label": "bod"
},
{
"id": 7,
"entity": "双链DNA",
"start_offset": 44,
"end_offset": 49,
"label": "bod"
},
{
"id": 8,
"entity": "整合酶",
"start_offset": 50,
"end_offset": 53,
"label": "bod"
},
{
"id": 9,
"entity": "宿主细胞",
"start_offset": 57,
"end_offset": 61,
"label": "bod"
},
{
"id": 10,
"entity": "染色体DNA",
"start_offset": 61,
"end_offset": 67,
"label": "bod"
},
{
"id": 11,
"entity": "前病毒DNA",
"start_offset": 72,
"end_offset": 78,
"label": "bod"
},
{
"id": 12,
"entity": "染色体",
"start_offset": 80,
"end_offset": 83,
"label": "bod"
}
] |
在人类，从黏膜感染到出现最初的病毒血症大约需要4～11天。 | [
{
"id": 0,
"entity": "黏膜感染",
"start_offset": 5,
"end_offset": 9,
"label": "dis"
},
{
"id": 1,
"entity": "病毒血症",
"start_offset": 15,
"end_offset": 19,
"label": "dis"
}
] |
经过短暂的高滴度病毒血症期后，病毒播散到全身淋巴组织。 | [
{
"id": 0,
"entity": "高滴度病毒血症",
"start_offset": 5,
"end_offset": 12,
"label": "dis"
},
{
"id": 1,
"entity": "病毒",
"start_offset": 15,
"end_offset": 17,
"label": "mic"
},
{
"id": 2,
"entity": "淋巴组织",
"start_offset": 22,
"end_offset": 26,
"label": "bod"
}
] |
由于机体的免疫应答，病毒复制被抑制，血中病毒含量迅速下降。 | [
{
"id": 0,
"entity": "病毒",
"start_offset": 10,
"end_offset": 12,
"label": "mic"
},
{
"id": 1,
"entity": "血",
"start_offset": 18,
"end_offset": 19,
"label": "bod"
},
{
"id": 2,
"entity": "病毒",
"start_offset": 20,
"end_offset": 22,
"label": "mic"
}
] |
此后，HIV感染分为两种不同的发展趋势，一种为快速进展，感染者迅速发病及死亡；另一种为缓慢进展，感染者进入慢性持续感染阶段。 | [
{
"id": 0,
"entity": "HIV感染",
"start_offset": 3,
"end_offset": 8,
"label": "dis"
}
] |
影响HIV感染向何种趋势发展的因素尚不清楚，可能与病毒的毒力、含量、宿主细胞辅助受体及免疫应答能力的差异有关。 | [
{
"id": 0,
"entity": "HIV",
"start_offset": 2,
"end_offset": 5,
"label": "mic"
},
{
"id": 1,
"entity": "病毒",
"start_offset": 25,
"end_offset": 27,
"label": "mic"
},
{
"id": 2,
"entity": "宿主细胞辅助受体",
"start_offset": 34,
"end_offset": 42,
"label": "bod"
}
] |
CD4+</sup>T淋巴细胞消耗的机制：HIV可感染CD4+</sup>T淋巴细胞以及表达CD4分子的单核/巨噬细胞和树突状细胞。 | [
{
"id": 0,
"entity": "T淋巴细胞",
"start_offset": 10,
"end_offset": 15,
"label": "bod"
},
{
"id": 1,
"entity": "HIV",
"start_offset": 21,
"end_offset": 24,
"label": "mic"
},
{
"id": 2,
"entity": "T淋巴细胞",
"start_offset": 37,
"end_offset": 42,
"label": "bod"
},
{
"id": 3,
"entity": "单核/巨噬细胞",
"start_offset": 52,
"end_offset": 59,
"label": "bod"
},
{
"id": 4,
"entity": "树突状细胞",
"start_offset": 60,
"end_offset": 65,
"label": "bod"
}
] |
病毒在易感细胞内复制、表达、整合并形成融合细胞，提示HIV可干扰或抑制细胞的正常功能，甚至对细胞造成直接损伤。 | [
{
"id": 0,
"entity": "病毒",
"start_offset": 0,
"end_offset": 2,
"label": "mic"
},
{
"id": 1,
"entity": "细胞",
"start_offset": 5,
"end_offset": 7,
"label": "bod"
},
{
"id": 2,
"entity": "细胞",
"start_offset": 21,
"end_offset": 23,
"label": "bod"
},
{
"id": 3,
"entity": "HIV",
"start_offset": 26,
"end_offset": 29,
"label": "mic"
},
{
"id": 4,
"entity": "细胞",
"start_offset": 35,
"end_offset": 37,
"label": "bod"
},
{
"id": 5,
"entity": "细胞",
"start_offset": 46,
"end_offset": 48,
"label": "bod"
}
] |
但是，针以HIV的特异性免疫应答，则是导致HIV感染细胞损伤的直接原因。 | [
{
"id": 0,
"entity": "HIV",
"start_offset": 5,
"end_offset": 8,
"label": "mic"
},
{
"id": 1,
"entity": "HIV感染细胞",
"start_offset": 21,
"end_offset": 28,
"label": "bod"
}
] |
被感染的CD4+</sup>T细胞由于表面表达HIV抗原，可被机体的细胞毒性T淋巴细胞（cytotoxicTlymphocyte，CTL）所识别和攻击，CTL清除病毒的同时破坏了大量受感染的CD4+</sup>T淋巴细胞。 | [
{
"id": 0,
"entity": "HIV抗原",
"start_offset": 23,
"end_offset": 28,
"label": "mic"
},
{
"id": 1,
"entity": "细胞毒性T淋巴细胞",
"start_offset": 34,
"end_offset": 43,
"label": "bod"
},
{
"id": 2,
"entity": "cytotoxicTlymphocyte",
"start_offset": 44,
"end_offset": 64,
"label": "bod"
},
{
"id": 3,
"entity": "CTL",
"start_offset": 65,
"end_offset": 68,
"label": "bod"
},
{
"id": 4,
"entity": "CTL",
"start_offset": 76,
"end_offset": 79,
"label": "bod"
},
{
"id": 5,
"entity": "病毒",
"start_offset": 81,
"end_offset": 83,
"label": "mic"
},
{
"id": 6,
"entity": "T淋巴细胞",
"start_offset": 105,
"end_offset": 110,
"label": "bod"
}
] |
但HIV具有高度变异性，虽然大量病毒被清除，变异的病毒株仍可存活下来，又侵入新生的CD4+</sup>T淋巴细胞，并在其中复制表达；再为CTL识别、攻击和清除，CD4+</sup>T淋巴细胞又大量被破坏。 | [
{
"id": 0,
"entity": "HIV",
"start_offset": 1,
"end_offset": 4,
"label": "mic"
},
{
"id": 1,
"entity": "病毒",
"start_offset": 16,
"end_offset": 18,
"label": "mic"
},
{
"id": 2,
"entity": "病毒",
"start_offset": 25,
"end_offset": 27,
"label": "mic"
},
{
"id": 3,
"entity": "T淋巴细胞",
"start_offset": 51,
"end_offset": 56,
"label": "bod"
},
{
"id": 4,
"entity": "CTL",
"start_offset": 68,
"end_offset": 71,
"label": "bod"
},
{
"id": 5,
"entity": "T淋巴细胞",
"start_offset": 90,
"end_offset": 95,
"label": "bod"
}
] |
如此周而复始，最后CD4+</sup>T淋巴细胞被消耗殆尽，形成继发性免疫缺陷，各种机会性病原体乘虚而入，在体内繁殖并引起疾病。 | [
{
"id": 0,
"entity": "T淋巴细胞",
"start_offset": 19,
"end_offset": 24,
"label": "bod"
},
{
"id": 1,
"entity": "继发性免疫缺陷",
"start_offset": 32,
"end_offset": 39,
"label": "dis"
}
] |
【临床表现】小儿感染HIV后，约经过5年左右的潜伏期才出现症状，绝大多数宫内HIV感染的婴儿在出生时无临床症状，体格检查正常。 | [
{
"id": 0,
"entity": "HIV",
"start_offset": 10,
"end_offset": 13,
"label": "mic"
},
{
"id": 1,
"entity": "宫内HIV感染",
"start_offset": 36,
"end_offset": 43,
"label": "dis"
}
] |
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